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AB164215

Recombinant Human CEP290 protein (GST tag N-Terminus)

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Recombinant Human CEP290 protein (GST tag N-Terminus) is a Human Fragment protein, in the 941 to 1104 aa range, expressed in Wheat germ, suitable for ELISA, WB.

View Alternative Names

BBS14, KIAA0373, NPHP6, CEP290, Centrosomal protein of 290 kDa, Cep290, Bardet-Biedl syndrome 14 protein, Cancer/testis antigen 87, Nephrocystin-6, Tumor antigen se2-2, CT87

1 Images
SDS-PAGE - Recombinant Human CEP290 protein (GST tag N-Terminus) (AB164215)
  • SDS-PAGE

Unknown

SDS-PAGE - Recombinant Human CEP290 protein (GST tag N-Terminus) (AB164215)

ab164215 on a 12.5% SDS-PAGE stained with Coomassie Blue.

Key facts

Expression system

Wheat germ

Tags

GST tag N-Terminus

Applications

WB, ELISA

applications

Biologically active

No

Accession

O15078

Animal free

No

Carrier free

No

Species

Human

Storage buffer

pH: 8 Constituents: 0.79% Tris HCl, 0.31% Glutathione

storage-buffer

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Complementary Products

Primary Antibodies

AB318279

Anti-CEP290 antibody [EPR28924-76] - BSA and Azide free

Recombinant|RabMAb

Immunocytochemistry/ Immunofluorescence - Anti-CEP290 antibody [EPR28924-76] - BSA and Azide free (AB318279)

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Proteins & Peptides

AB159886

Recombinant Human ZP2 protein (GST tag N-Terminus)

SDS-PAGE - Recombinant Human ZP2 protein (GST tag N-Terminus) (AB159886)

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  • 0
Proteins & Peptides

AB162402

Recombinant Human Zinc finger protein 691 (GST tag N-Terminus)

SDS-PAGE - Recombinant Human Zinc finger protein 691 (GST tag N-Terminus) (AB162402)

  • 0
  • 0
Proteins & Peptides

AB161146

Recombinant Human BS69 protein (GST tag N-Terminus)

SDS-PAGE - Recombinant Human BS69 protein (GST tag N-Terminus) (AB161146)

  • 0
  • 0
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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "ELISA": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" }, "WB": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }
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Sequence info

[{"sequence":"MAIFKIAALQKVVDNSVSLSELELANKQYNELTAKYRDILQKDNMLVQRTSNLEHLECENISLKEQVESINKELEITKEKLHTIEQAWEQETKLGNESSMDKAKKSITNSDIVSISKKITMLEMKELNERQRAEHCQKMYEHLRTSLKQMEERNFELETKFAEV","proteinLength":"Fragment","predictedMolecularWeight":null,"actualMolecularWeight":null,"aminoAcidEnd":1104,"aminoAcidStart":941,"nature":"Recombinant","expressionSystem":"Wheat germ","accessionNumber":"O15078","tags":[{"tag":"GST","terminus":"N-Terminus"}]}]
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Properties and storage information

Shipped at conditions
Dry Ice
Appropriate short-term storage conditions
-80°C
Appropriate long-term storage conditions
-80°C
Aliquoting information
Upon delivery aliquot
Storage information
Avoid freeze / thaw cycle
False
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Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

CEP290 also known as centrosomal protein of 290 kDa acts as an important structural component in cells. It has an approximate mass of 290 kilodaltons and is primarily expressed in ciliated cells across various tissues including the retina and kidneys. CEP290 localizes at the centrosome and is integral to the formation and stability of cilia and flagella. The protein plays an important mechanic role in maintaining the structural integrity of cilia which are essential for cellular signaling and motility.
Biological function summary

CEP290 contributes significantly to the formation and maintenance of the primary cilium a cellular organelle. It forms part of the transition zone complex a critical region at the base of cilia. This zone functions as a barrier regulating the movement of molecules in and out of the cilia. As a result CEP290 supports the transport and localization of ciliary proteins affecting ciliary homeostasis and signal transduction mechanisms within the cell.

Pathways

CEP290 is intricately involved in the ciliary transport and the phototransduction pathway within photoreceptor cells. CEP290 operates in coordination with proteins such as RPGR in these pathways ensuring proper ciliary trafficking and function. This activity is important for sensory perception processes including vision. The disruption of CEP290 affects these pathways leading to compromised sensory functions.

CEP290 mutations associate with specific ciliopathies such as Joubert syndrome and Leber congenital amaurosis. These conditions result from defective ciliary function and altered cellular pathways. In the context of Joubert syndrome CEP290 interacts with proteins such as AHI1 where both play roles in neurodevelopment and function. The understanding of these interactions provides insights into the mechanistic implications of CEP290-related disorders.
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Specifications

Form

Liquid

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General info

Function

Involved in early and late steps in cilia formation. Its association with CCP110 is required for inhibition of primary cilia formation by CCP110 (PubMed : 18694559). May play a role in early ciliogenesis in the disappearance of centriolar satellites and in the transition of primary ciliar vesicles (PCVs) to capped ciliary vesicles (CCVs). Required for the centrosomal recruitment of RAB8A and for the targeting of centriole satellite proteins to centrosomes such as of PCM1 (PubMed : 24421332). Required for the correct localization of ciliary and phototransduction proteins in retinal photoreceptor cells; may play a role in ciliary transport processes (By similarity). Required for efficient recruitment of RAB8A to primary cilium (PubMed : 17705300). In the ciliary transition zone is part of the tectonic-like complex which is required for tissue-specific ciliogenesis and may regulate ciliary membrane composition (By similarity). Involved in regulation of the BBSome complex integrity, specifically for presence of BBS2, BBS5 and BBS8/TTC8 in the complex, and in ciliary targeting of selected BBSome cargos. May play a role in controlling entry of the BBSome complex to cilia possibly implicating IQCB1/NPHP5 (PubMed : 25552655). Activates ATF4-mediated transcription (PubMed : 16682973).

Post-translational modifications

Ubiquitinated. May undergo monoubiquitination; monoubiquitination is inhibited in response to cellular stress, such as ultraviolet light (UV) radiation or heat shock, but does not cause its displacement from centriolar satellites.

Subcellular localisation

Cytoskeleton

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Product protocols

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Target data

Involved in early and late steps in cilia formation. Its association with CCP110 is required for inhibition of primary cilia formation by CCP110 (PubMed : 18694559). May play a role in early ciliogenesis in the disappearance of centriolar satellites and in the transition of primary ciliar vesicles (PCVs) to capped ciliary vesicles (CCVs). Required for the centrosomal recruitment of RAB8A and for the targeting of centriole satellite proteins to centrosomes such as of PCM1 (PubMed : 24421332). Required for the correct localization of ciliary and phototransduction proteins in retinal photoreceptor cells; may play a role in ciliary transport processes (By similarity). Required for efficient recruitment of RAB8A to primary cilium (PubMed : 17705300). In the ciliary transition zone is part of the tectonic-like complex which is required for tissue-specific ciliogenesis and may regulate ciliary membrane composition (By similarity). Involved in regulation of the BBSome complex integrity, specifically for presence of BBS2, BBS5 and BBS8/TTC8 in the complex, and in ciliary targeting of selected BBSome cargos. May play a role in controlling entry of the BBSome complex to cilia possibly implicating IQCB1/NPHP5 (PubMed : 25552655). Activates ATF4-mediated transcription (PubMed : 16682973).
See full target information CEP290
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